Here, we focus on the PIDDs commonly presented with atopic dermatitis-like skin lesions, including hyper-immunoglobulin E (IgE) syndromes (HIES), Wiskott-Aldrich syndrome (WAS), immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, STAT5B deficiency, Omenn syndrome (OS), atypical complete DiGeorge syndrome, and X-linked agammaglobulinemia (XLA). This evidence concerns the gene STAT5B and hyperinsulinemic hypoglycemia, familial, 4.